Massive Splenomegaly
Publication: Annals of Internal Medicine: Clinical Cases
Volume 2, Number 2
Submit a Comment
Contributors must reveal any conflict of interest. Comments are moderated. Please see our information for authorsregarding comments on an AIMCC publication.
Abstract
Massive splenomegaly, defined as length spleen greater than 20 cm, is a relatively rare entity with a broad differential. Primary splenomegaly occurs in the setting of leukemias and lymphomas, whereas secondary splenomegaly is more common and carries a broad differential. We present a case of massive splenomegaly with an approach to differential diagnoses and brief discussion of splenic marginal zone lymphoma.
A 40-year-old female patient presented with a 1-month history of progressive abdominal fullness and anorexia. She did not report associated symptoms, such as nausea/vomiting, skin yellowing, and changes in the urine/stool. She did not report alcohol intake. Computed tomography of the abdomen revealed massive splenomegaly (30.55 cm) (Figure 1). Significant laboratory values included hemoglobin 10.8 g/dL, platelets 118 ×103/mcL, leukocyte count 4.9 ×103/mcL, and normal liver function.
Splenomegaly is defined by length via imaging, with >20 cm categorized as massive splenomegaly. Massive primary splenomegaly involves proliferation of native splenic cells, usually in the setting of leukemia or lymphoma (Table 1) (1). Secondary splenomegaly is more common and carries a wide differential that includes congestion, hyperplasia, and infiltration (Table 2) (2, 3). Splenomegaly commonly presents with nonspecific abdominal discomfort. Therefore, history and physical examination are necessary to narrow the broad differential. Further testing should be guided by specific differentials. Imaging should include ultrasonography to evaluate splenic echogenicity/texture and computed tomography to examine surrounding structures, including lymph nodes. In suspected malignancy, evaluation includes peripheral blood smear, flow cytometry, and immunochemical assays. Blood cultures and bone marrow examination should be immediately pursued in patients who are clinically ill (4).
| Lymphomas | • Diffuse large B-cell lymphoma • Splenic marginal zone lymphoma • Mantle cell lymphoma • Waldenstrom macroglobulinemia |
| Leukemias | • Chronic myeloid leukemia • Hairy cell leukemia • Chronic lymphocytic leukemia • Acute lymphoblastic leukemia |
| Type | Pathophysiology | Differential Diagnosis |
|---|---|---|
| Congestive | Pooling of blood or failed drainage | • Portal hypertension in liver failure • Chronic heart failure • Venous thrombosis of portal or hepatic veins |
| Splenic sequestration | • Sickle cell disease • Hemolytic anemia • Thalassemia • Felty syndrome | |
| Infiltrative | Autoimmune disease | • Systemic lupus erythematosus • Rheumatoid arthritis • Still disease • Dermatomyositis • Scleroderma • Hemophagocytic lymphohistiocytosis |
| Deposition disease | • Sarcoidosis • Amyloidosis • Glycogen storage diseases • Hemochromatosis | |
| Nonhematologic malignancy | • Melanoma • Primary breast, lung, ovarian, stomach, and prostate cancers | |
| Hyperplasia | Infection with reticuloendothelial cell hyperplasia | • Viral: mononucleosis, tuberculosis, HIV • Parasitic: histiocytosis, malaria, leptospirosis • Bacterial: mycobacteria, persistent bacteremia |
| Bone marrow failure with extramedullary hematopoiesis | • Primary myelofibrosis • Polycythemia vera • Hemolysis |
In this patient, bone marrow biopsy confirmed splenic marginal zone lymphoma. Splenic marginal zone lymphoma is an indolent B-cell malignancy often associated with hepatitis C and may be treated with viral eradication, although this patient tested negative. Therapy involves single-agent rituximab, splenectomy, or chemotherapeutics. Further therapies are derived from those used in indolent non-Hodgkin lymphoma (5). This patient was initiated on rituximab-based therapy. However, as the result of minimal improvement at follow-up, she began evaluation for splenectomy.
Supplemental Material
References
1.
Thipphavong S, Duigenan S, Schindera ST, et al. Nonneoplastic, benign, and malignant splenic diseases: cross-sectional imaging findings and rare disease entities. AJR Am J Roentgenol. 2014;203:315-22. [PMID: 25055265] doi: 10.2214/AJR.13.11777
2.
Chapman J, Bansal P, Goyal A, et al. Splenomegaly. In: StatPearls. Treasure Island (FL): StatPearls Publishing; 2021. Accessed 15 April 2022. https://www.ncbi.nlm.nih.gov/books/NBK430907/
3.
Jacob H. Splenomegaly. Merck Manual. Accessed 15 April 2022. https://www.merckmanuals.com/professional/hematology-and-oncology/spleen-disorders/splenomegaly
4.
Pozo AL, Godfrey EM, Bowles KM. Splenomegaly: investigation, diagnosis and management. Blood Rev. 2009;23:105-11. [PMID: 19062140] doi: 10.1016/j.blre.2008.10.001
5.
Arcaini L, Rossi D, Paulli M. Splenic marginal zone lymphoma: from genetics to management. Blood. 2016;127:2072-81. [PMID: 26989207] doi: 10.1182/blood.2015.11.624312
Information & Authors
Information
Published In
Annals of Internal Medicine: Clinical Cases
Volume 2 • Number 2 • February 2023
History
Published in issue: February 2023
Published online: 7 February 2023
Keywords
Copyright
© 2023 Authors. Published in partnership by the American College of Physicians and American Heart Association. This is an open access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND), which allows reusers to copy and distribute the material in any medium or format in unadapted form only, for noncommercial purposes only, and only so long as attribution is given to the creator. See: https://creativecommons.org/licenses/by-nc-nd/4.0/legalcode.
Authors
Metrics & Citations
Metrics
Citations
If you have the appropriate software installed, you can download article citation data to the citation manager of your choice. For an editable text file, please select Medlars format which will download as a .txt file. Simply select your manager software from the list below and click Download.
For more information or tips please see 'Downloading to a citation manager' in the Help menu.
Download article citation data for:
Massive Splenomegaly. AIM Clinical Cases.2023;2:e220444. [Epub 7 February 2023]. doi:10.7326/aimcc.2022.0444
Massive Splenomegaly. AIM Clinical Cases.2023;2:e220444. [Epub 7 February 2023]. doi:10.7326/aimcc.2022.0444
View More
View options
PDF/ePub
View PDF/ePubGet Access
Login Options:
Purchase
You will be redirected to acponline.org to sign-in to Annals to complete your purchase.
Access to EPUBs and PDFs for FREE Annals content requires users to be registered and logged in. A subscription is not required. You can create a free account below or from the following link. You will be redirected to acponline.org to create an account that will provide access to Annals. If you are accessing the Free Annals content via your institution's access, registration is not required.
Create your Free Account
You will be redirected to acponline.org to create an account that will provide access to Annals.
Comment on Massive Splenomegaly
This case represented a dramatic example of massive splenomegaly.
The classification of splenomegaly was, however, a bit off. The groups Lymphoma and Leukemia, should have been instead Lymphoid Neoplasms and Hematopoietic (Myeloid) Neoplasms to jibe with the World Health Organization Classification. In so doing, acute and chronic lymphocytic leukemia should be listed under Lymphoid Neoplasms. Polycythemia vera and Primary Myelofibrosis should be shown under Hematopoietic (Myeloid) Neoplasms. Primary myelofibrosis, which in my view is better called chronic megakaryocytic leukemia, does not have effective extramedullary hematopoiesis, established by the absence of any deterioration in blood counts if a massive spleen is excised. The blood counts either remain the same or improve. Because of the very high concentration of circulating CD34 cells, the numerous immature myeloid cells in spleen, are more likely akin to metastasis being trapped in spleen or liver or other sites and, possibly, differentiating into precursor cells.(1)
Hemolysis, of course, is not a marrow failure syndrome. In acute hemolysis, erythropoiesis can increase three-fold and in chronic hemolysis can increase six-fold. There may be extramedullary islands of erythroblasts in some chronic hemolytic states, but they are of no functional significance. They may, for example, if in enlarged mediastinal lymph nodes, create a diagnostic problem of seemingly unexplained lymphadenopathy on rare occasions. This event may happen in other sites as well.
An interesting case.
REFERENCES
(1) Lichtman MA. Is it primary myelofibrosis or chronic megakaryocytic leukemia? Haematologica. 2022 Dec 1;107(12):2779-2781. doi: 10.3324/haematol.2022.280838. PMID: 35295083; PMCID: PMC9713543.
Follow-up comment
The designation of one category of splenomegaly as “Infection with reticuloendothelial cell hyperplasia” is an obsolete misnomer. The term “reticuloendothelial system” was coined by Aschoff in 1924 to describe the group of cells that had the ability to incorporate vital dyes from the circulation. Although this was a conceptual leap forward 99 years ago, the cells were neither reticular nor endothelial. The system is now referred to as the “monocyte-macrophage system” or the “mononuclear phagocyte system”. The designation “reticuloendothelial system” has been obsolete for decades and should be allowed to rest in peace. It does not represent an accurate designation of the cells composing this extensive system.